The Agenesis of the Right Lobe of a Liver with Multiple Intrahepatic Bile Duct Stones: A case report

Roh, Jung Ho; Cho, Chol Kyoon; Kim, Hyun Jong

Abstract

Agenesis of the right lobe of the liver is such a rare congenital anomaly that only about 40 cases have been reported in the literature. This anomaly is considered to be caused by a developmental failure of the right portal vein or by an error of mutual induction between the primitive diaphragm and the endodermal diverticulum representing the primitive liver. When the absence or hypoplasia of the right lobe is found by radiological examinations, several condition, such as liver cirrhosis, a cholangiocarcinoma, and a previous hepatic resection, must be taken into a consideration for a differential diagnosis besides agenesis. Agenesis of the right lobe could be complicated by various biliary tract diseases, including cholelithiasis, a carcinoma of the gallbladder and Mirizzi syndrome, portal hypertension, and rarely volvulus of the stomach, and these complications usually draw clinical attention. We experienced a 58-year-old female who complained of intermittent fever, chills, and jaundice and who was diagnosed with agenesis of the right lobe of the liver with multiple intrahepatic stones by using abdominal CT and MRI. Percutaneous transhepatic biliary drainage was done to relieve the obstructive jaundice, and a percutaneous transhepatic cholangiogram revealed agenesis of the right lobe and abnormal course of the biliary tract. In operative findings, there was no right lobe, and the left lateral segment was markedly enlarged with compensatory hypertrophy. However, the medial segment of the left lobe and caudate lobe was relatively normal in size. The gallbladder was located on the right side of the liver retrohepatically. A cholecystectomy and a choledocholithotomy through a choledochotomy and a retrograde approach after a subsegmentectomy of segments II and III were done.